Sahota Preeti, Das Chandi P, Prabhakar Sudesh, Rajwanshi A, Singh Paramjeet, Khurana Dheeraj
From the Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
J Clin Neuromuscul Dis. 2005 Sep;7(1):29-35. doi: 10.1097/01.cnd.0000176975.38265.05.
Peripheral neuropathy is usually the presenting feature of POEMS syndrome. Approximately 50% of patients with POEMS syndrome are associated with osteosclerotic myeloma, a rare variant of multiple myeloma, and some with Castleman's disease, an unusual lymphoproliferative disorder. The multicentric plasma cell variant of Castleman's disease is usually associated with systemic disorders rather than its localized form of mediastinal lymphoid hyperplasia characterized by hyalinization of follicles and interfollicular vascular proliferation. We report a 48-year-old woman who presented with progressive sensorimotor demyelinating polyradiculoneuropathy, bilateral optic disc edema, hepatosplenomegaly, generalized lymphadenopathy, and skin changes. There was associated thrombocytosis, hypothyroidism, hypoparathyroidism, mixed osteolytic and osteosclerotic bone lesions, monoclonal gammopathy of IgG lambda type, and hyaline vascular type of Castleman's disease. This combination of POEMS syndrome, osteosclerotic myeloma with a hyaline vascular type of Castleman's disease is uncommon.
周围神经病变通常是POEMS综合征的首发症状。约50%的POEMS综合征患者与骨硬化性骨髓瘤（一种罕见的多发性骨髓瘤变异型）相关，部分患者与Castleman病（一种罕见的淋巴增殖性疾病）有关。Castleman病的多中心浆细胞变异型通常与全身性疾病相关，而非其以滤泡透明变性和滤泡间血管增生为特征的局限性纵隔淋巴样增生形式。我们报告了一名48岁女性，她表现为进行性感觉运动性脱髓鞘性多神经根神经病、双侧视盘水肿、肝脾肿大、全身淋巴结肿大及皮肤改变。同时伴有血小板增多症、甲状腺功能减退、甲状旁腺功能减退、混合性溶骨性和骨硬化性骨病变、IgG λ型单克隆丙种球蛋白病以及透明血管型Castleman病。POEMS综合征、骨硬化性骨髓瘤合并透明血管型Castleman病的这种组合并不常见。
